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J Neurol Neurosurg Psychiatry 83:488-494 doi:10.1136/jnnp-2011-302029
  • Neuro-ophthalmology
  • Review

Update on the pathophysiology and management of idiopathic intracranial hypertension

Editor's Choice
  1. Nancy J Newman1,2,3
  1. 1Department of Ophthalmology, Emory University, Atlanta, Georgia, USA
  2. 2Department of Neurology, Emory University, Atlanta, Georgia, USA
  3. 3Department of Neurological Surgery, Emory University, Atlanta, Georgia, USA
  1. Correspondence to Dr V Biousse, Neuro-Ophthalmology Unit, Emory Eye Center, 1365-B Clifton Road NE, Atlanta, GA 30322, USA; vbiouss{at}emory.edu
  1. Contributors All three authors contributed to the review of the literature, and the preparation of the manuscript and figures.

  • Received 13 December 2011
  • Revised 20 January 2012
  • Accepted 25 January 2012
  • Published Online First 15 March 2012

Abstract

Idiopathic intracranial hypertension is a disease of unknown aetiology, typically affecting young obese women, producing a syndrome of increased intracranial pressure without identifiable cause. Despite a large number of hypotheses and publications over the past decade, the aetiology is still unknown. Vitamin A metabolism, adipose tissue as an actively secreting endocrine tissue and cerebral venous abnormalities are areas of active study regarding the pathophysiology of idiopathic intracranial hypertension. There continues to be no evidence based consensus or formal guidelines regarding management and treatment of the disease. Treatment studies show that the diagnostic lumbar puncture is a valuable intervention beyond its diagnostic importance, and that weight management is critical. However, many questions remain regarding the efficacy of acetazolamide, CSF shunting procedures and cerebral transverse venous sinus stenting.

Footnotes

  • Funding This study was supported in part by a departmental grant (Department of Ophthalmology) from Research to Prevent Blindness Inc, New York, by core grant P30-EY06360 (Department of Ophthalmology). BBB receives research support from the NIH/PHS (KL2-RR025009, UL1-RR025008), NIH/NEI (K23-EY019341) and the Knights Templar Eye Foundation; and received the American Academy of Neurology Practice Research Fellowship. NJN is a recipient of the Research to Prevent Blindness Lew R Wasserman Merit Award.

  • Competing interests None.

  • Provenance and peer review Commissioned; externally peer reviewed.

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